CORRECT DIAGNOSIS:

Anaplastic Large Cell Lymphoma

DISCUSSION:

CD30+ T-cell lymphomas encompass a group of lymphoproliferative disorders that primarily include anaplastic large cell lymphoma (ALCL) variants, categorized into ALK-1 positive ALCL, ALK-1 negative ALCL, and breast implant-associated ALCL, which is recognized as a provisional entity. These lymphomas represent approximately 3% of adult non-Hodgkin lymphomas but account for a significantly higher proportion, around 10-20%, of pediatric lymphomas. The demographic most affected typically falls between the ages of 45 and 60, with a notable male predominance.

The ALK-1 positive ALCL subtype of CD30+ T-cell lymphoma is distinguished by the presence of the ALK-1 gene rearrangement, which is commonly associated with a more favorable prognosis. Patients with ALK-1 positive ALCL often exhibit variable genetic and clinical features. The 5-year survival rate for these patients is significantly high, ranging from 70% to 80%. ALK-1 negative ALCL subtype is typically observed in primary cutaneous cases, this variant tends to manifest as localized disease and generally carries a good prognosis. However, ALK-1 negative systemic ALCL presents a more challenging clinical scenario, with a poorer prognosis. The 5-year survival rate for this group is considerably lower, at 33% to 49%. Breast Implant-Associated ALCL is a newly recognized entity that has garnered attention due to its association with breast implants. While it shares characteristics with other forms of ALCL, its clinical context and unique presentation distinguish it as a separate category within CD30+ lymphomas. 

Systemic ALCL is frequently characterized by “B symptoms,” which include fever, fatigue, weight loss, and night sweats. Patients may also exhibit respiratory symptoms such as cough and dyspnea due to mediastinal lymph node involvement. Furthermore, extralymphatic site involvement can occur in various organs, including bones, bone marrow, subcutaneous tissues, lungs, spleen, and liver

Diagnosis of CD30+ T-cell lymphomas is multifaceted, incorporating clinical evaluation, imaging studies, and histopathological examination. In our case, histopathological findings indicated a CD30+ lymphoproliferative disorder, favoring anaplastic large cell lymphoma (likely primary cutaneous ALCL) over lymphomatoid papulosis type C. This distinction is crucial for guiding treatment and management strategies, as the clinical and immunophenotypic profiles significantly influence the prognosis.

TREATMENT:

CONCLUSION:

CD30+ T-cell lymphomas, particularly anaplastic large cell lymphoma, encompass a diverse group of malignancies with varying clinical presentations and prognoses.The management of CD30+ T-cell lymphomas requires a comprehensive approach tailored to the specific lymphoma subtype and patient characteristics. While ALK-1 positive cases typically respond well to anthracycline-based chemotherapy and targeted therapies, ALK-1 negative and breast implant-associated ALCL may necessitate different strategies, including surgical excision and radiation therapy. The ongoing evaluation of treatment responses is crucial for optimizing patient outcomes and ensuring comprehensive care.

REFERENCES:

Connors, J. M., et al. (2018). Brentuximab vedotin combined with chemotherapy for stage III and IV anaplastic large-cell lymphoma: A phase II study. Journal of Clinical Oncology, 36(31), 3177-3185. https://doi.org/10.1200/JCO.2018.79.9203 [PMID: 30220953]

Hutchinson, C. B., & Mott, R. D. (2020). Anaplastic large cell lymphoma: A clinical and pathologic overview. American Journal of Clinical Pathology, 154(5), 668-676. https://doi.org/10.1093/ajcp/aqaa106 [PMID: 32926365]

Swerdlow, S. H., Campo, E., Harris, N. L., et al. (2016). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. International Agency for Research on Cancer.

De Jong, D., Mollema, F. P., & van der Lee, J. (2021). Breast implant-associated anaplastic large cell lymphoma: A review. Current Oncology Reports, 23(4), 1-9. https://doi.org/10.1007/s11912-021-01062-3 [PMID: 33717234]